Title :

Episodic angioedema with eosinophilia (gleich syndrome) in children: a clinical review

Author(s) :

Bertrand, Valerie [Auteur]
Boccara, Olivia [Auteur]
Filhon, Bruno [Auteur]
Manca, Florian [Auteur]
Lefevre, Guillaume [Auteur]
Groh, Matthieu [Auteur]
Kahn, Jean-Emmannuel [Auteur]

Journal title :

Pediatric allergy and immunology . official publication of the European Society of Pediatric Allergy and Immunology

Abbreviated title :

Pediatr Allergy Immunol

Publication date :

2019-11-14

ISSN :

1399-3038

English keyword(s) :

children
Gleich syndrome
episodic angioedema with eosinophilia

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

Episodic angioedema with eosinophilia (EAE, Gleich syndrome) is a rare disease, consisting of recurrent angioedema with hypereosinophilia and frequent increased serum immunoglobulin M levels. Less than 100 patients have ...
Show more >Episodic angioedema with eosinophilia (EAE, Gleich syndrome) is a rare disease, consisting of recurrent angioedema with hypereosinophilia and frequent increased serum immunoglobulin M levels. Less than 100 patients have been reported, mainly adults, sometimes with underlying lymphocytic variant of hypereosinophilic syndrome (HESL We performed a retrospective study of all pediatric cases of EAE referred within the French National Referral Center for Hypereosinophilic Syndrome (CEREO). Next, the PRISMA guidelines were applied in order to perform a systematic review (data sources: PubMed, Web of Science). Among the two reported and 15 previously published cases of EAE occurring in children, the main clinical findings mimicked those of adults, including recurrent angioedema, hives, and weight gain. The median time between the first angioedema flare and the diagnosis of EAE was 5 years in published cases. Hypereosinophilia was constant, usually worsening with each attack, but seldom disappeared between flares. Total IgM serum levels were elevated in 16 patients. Four children had evidence of abnormal CD3-+ Pediatricians should be aware that EAE is a diagnosis to consider in children. T-cell immunophenotyping is warranted in this setting. Prognosis seems fair, yet eosinophil-related organ damage may occur in patients with persistent eosinophilia.Show less >

Language :

Anglais

Audience :

Internationale

Popular science :

Non

Administrative institution(s) :

CHU Lille
Inserm
Université de Lille

Collections :

• Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286

Submission date :

2024-01-30T10:28:29Z